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Hydronephrosis of Pregnancy in an Ectopic Pelvic Kidney

We present a case of a woman with an ectopic pelvic kidney and symptomatic hydronephrosis who failed conservative treatment and required ureteric stent placement.

Authors: Toepfer, Nicholas; Parikh, Ankur; Bhangdia, Darshan
Corresponding Author: Toepfer, Nicholas J

 

Introduction
Hydronephrosis during pregnancy is common and has a reported occurrence between 43% and 100% (1).  Both mechanical and hormonal changes during pregnancy are thought to contribute to its etiology, but evidence suggests it is mainly due to compression of the ureters between the pregnant uterus and the linea terminalis (2,3). It has been postulated that this phenomenon does not affect women whose ureters do not cross the pelvic brim. The incidence of an ectopic pelvic kidney is approximately 1 in 2200 to 3000 patients (4,5). Ectopic kidneys occur due to failure of the kidney to ascend during weeks 6-9 of fetal development. Although ectopic pelvic kidneys are relatively common, only one case of hydronephrosis during pregnancy in a pelvic kidney is described in the literature (6). This supports the notion that hydronephrosis during pregnancy in pelvic kidneys is exceedingly rare. We present a case of a woman with an ectopic pelvic kidney and symptomatic hydronephrosis who failed conservative treatment and required ureteric stent placement.

Case Presentation
A 31 year old pregnant female at 22 weeks gestation presented with complaints of sharp right lower quadrant pain, nausea, and vomiting. She denied any lower urinary tract symptoms. On physical examination, she was afebrile and haemodynamically stable. The gravid abdomen was soft, non-tender and no pain could be elicited on palpation. Her urinalysis was negative for leukocyte esterase and nitrite. She demonstrated a leukocytosis of 12.7 K/µL with 88% segmented neutrophils and 7% lymphocytes. A CT scan of the abdomen and pelvis with intravenous contrast was performed for suspected acute appendicitis. The CT scan showed an ectopic right kidney located in the pelvis with mild hydronephrosis of an anteriorly oriented collecting system (Figure 1a-b). The ectopic kidney was malrotated, medially deviated, and located anterior to the iliac bifurcation and immediately posterior to the gravid uterus. The left kidney was unremarkable and located in the orthotopic location in the left retroperitoneum. The patient’s severe right lower quadrant pain persisted despite intravenous analgesia. The patient was transferred to a tertiary medical center for further care.
The patient was counseled on the options regarding symptomatic hydronephrosis during pregnancy and she opted for conservative management with intravenous and oral analgesia. She continued to experience intractable abdominal pain and after 48 hours of medical management, she decided to undergo a cystoscopy with retrograde ureteric stent placement.
At operation, there were no abnormal findings on cystoscopy. A retrograde pyelogram was performed. The ureter had no evidence of filling defects as it coursed to a midline kidney located at the level of the L4 vertebral body. An 18 cm long 4.7 Fr diameter stent was placed and the location was confirmed using fluoroscopy. Postoperatively, the patient’s pain completely resolved but she did report mild bladder irritation, frequency and urgency from the ureteric stent.
The patient underwent exchange of her stent 12 weeks later. She eventually underwent a caesarean section for a breech presentation at 39 weeks. This was completed without complications and the newborn baby was without any identifiable abnormalities. The ureteric stent was removed 12 days later and the patient remained asymptomatic afterwards. A renal ultrasound one month after stent removal demonstrated resolution of the hydronephrosis (Figure 2).

Discussion
Hydronephrosis can manifest at different stages during pregnancy. A large prospective study found hydronephrosis present in 15%, 20% and 50% of women during the first, second, and third trimesters respectively.1 The development of dilatation at various stages of pregnancy may represent different etiologies of hydronephrosis in pregnancy including mechanical and hormonal effects.
The 15% incidence of ureteric dilation on ultrasound during the first trimester occurs before the uterus reaches the pelvic brim. This supports a non-mechanical etiology for the development of hydronephrosis. Increased levels of progesterone during pregnancy have been hypothesized to promote ureteric dilatation and inhibit peristalsis which causes subsequent dilation of the upper urinary tract (1,7). In addition, progesterone has been shown to slow the rate of disappearance of hydroureter in postpartum women (8).
While hormonal changes may contribute to hydronephrosis of pregnancy, the most plausible etiologic factor is mechanical obstruction of the ureter by the gravid uterus. Studies have shown normal ureteral contractile pressures in pregnant women suggesting that hormonal induced atony is not the primary factor in ureteric dilation (2). Additionally, women whose ureters do not cross the pelvic brim, such as those associated with ectopic and pelvic kidneys or non-orthotopic urinary diversion, have been shown not to develop ureteric dilation of pregnancy (9-11). Despite this evidence, our patient demonstrates that women with pelvic kidneys can develop hydronephrosis during pregnancy.
Pelvic kidneys present challenges in treatment for a variety of reasons including tortuosity of the ureters limiting endoscopic access, as well as a greater risk of injuring aberrant vessels or overlying abdominal viscera. Management of symptomatic hydronephrosis in pregnancy is often treated with ureteric stent placement (12-15). Advantages of treating an obstructed kidney with a percutaneous nephrostomy rather than an indwelling ureteric stent are the lower cost and ease of changes during pregnancy (16). In addition, percutaneous nephrostomies have a lower incidence of irritative LUTS, reduced analgesic requirements and patients are thought to have a better quality of life as compared to those patients with indwelling stents (17,18). In this particular patient, the location of the ectopic kidney was such that a percutaneous nephrostomy could not be placed safely under ultrasound guidance due to proximity of the iliac artery. In order to minimize the risk of radiation exposure to the fetus, the ALARA principle was applied. The fluoroscopy settings during stent placement were set to low dose with collimation and the patient’s gravid abdomen was shielded using a lead gown. Fluoroscopy time was limited as much as possible and there was only a total accumulated dose area product to the patient of 0.7 Gy/cm2.
Any surgery during pregnancy has increased risks. Pregnancy is a hypercoagulable state associated with an increased risk of deep venous thrombosis and thromboembolism and there is also an increased risk of aspiration during general anesthesia. Regarding potential risks to the fetus during surgery, a review of 5,405 patients found no increased incidence of congenital abnormalities, premature labor or stillbirths during non-obstetrical operations (19). Rapid encrustation of ureteric stents as a result of the hypercalciuria and hyperuricosuria during pregnancy must be considered when deciding when to exchange stents (20). This patient had no prior history of urolithiasis so it was decided to extend the time to stent exchange closer to her due date at 34 weeks gestation in case the endoscopic manipulation induced premature labor.
Resolution of the hydronephrosis following delivery confirmed that the obstruction was secondary to the pregnancy and not a previously undiagnosed chronic obstruction such as pelviureteric junction obstruction which is common in ectopic kidneys (4).

Conclusion
Hydronephrosis of pregnancy occurs in most women but it has been found not to affect those patients with pelvic kidneys. This patient illustrates that it is possible for women with ectopic pelvic kidneys to develop hydronephrosis during pregnancy. While conservative management is successful in controlling symptoms in most patients with hydronephrosis during pregnancy, a patient with a pelvic kidney such as this may require more aggressive drainage of the kidney with a ureteric stent or nephrostomy.

 

Toepfer-Fig1a

Fig.1a

Toepfer-Fig1b

Fig1.b

Toepfer-Fig1b

Fig.2

Acknowledgments
None

Disclosure Statement
No competing financial interests exist.

References
1. Faundes A, Bricola-Filho M, Pinto et al. Dilatation of the urinary tract during pregnancy: Proposal of a curve of maximal caliceal diameter by gestational age. Am J Obstet Gynecol 1998; 178(5):1082-1086.
2. Roberts JA. Hydronephrosis of pregnancy. Urology 1976; 8(1):1-4.
3. Rasmussen PE, Nielsen FR. Hydronephrosis during pregnancy: a literature survey. Eur J Obstet Gynecol Reprod Biol 1988; 27(3):249-259.
4. Cinman NM, Okeke Z, Smith AD. Pelvic Kidney: Associated Diseases and Treatment. J Endourology 2007; 21(8):836-842.
5. Zafar FS, Lingeman JE. Value of laparoscopy in the management of calculi complicating renal malformations. J Endourol 199; 10(4):379-383.
6. Malhorta N, Roy KK, Garg PK, et al. Ectopic hydronephrotic kidney masquerading as an ovarian cyst during pregnancy. Eur J Obstet Gynecol Reprod Biol 2001; 97(2):239-240.
7. van Wagenen G, Jenkins RH. An experimental examination of factors causing ureteral dilation of pregnancy. J Urol 1939; 42:1010-1020.
8. Lubin S, Drexler LS, Bilotta WA. Post-partum pyeloureteral changes following hormone administration. Surg Gynecol Obstet 1941; 73:391.
9. Swanson SK, Heilman RL, Eversman WG. Urinary tract stones in pregnancy. Surg Clin North Am 1995; 75:123-142.
10. McAleer SJ, Loughlin KR. Nephrolithiasis and pregnancy. Curr Opin Urol 2004; 14:123-127.
11. Harrow BR, Sloane JA, Salhanith L. Etiology of hydronephrosis of pregnancy. Surg Gynecol Obstet 1964; 119:1042-1048.
12. Sadan O, Berar M, Sagiv R, et al. Ureteric stent in severe hydronephrosis of pregnancy. Eur J Obstet Gynecol Reprod Biol 1994; 56(2):79-81.
13. Delakas D, Karyotis I, Loumbakis P, et al. Ureteral drainage by Double-J-catheters during pregnancy. Clin Exp Obstet Gynecol 2000; 27(3-4):200-202.
14. Fainaru O, Almog B, Gamzu R, et al. The Management of Symptomatic Hydronephrosis in pregnancy. BJOG 2002; 109:1385-1387.
15. Vendola N, Giumelli P, Galdini R, et al. Ureteral Drainage with Double-J Catheters in Obstructive Uropathy during Pregnancy. Gynecol Obstet Invest 1995; 40:274-275.
16. Kavoussi LR, Albala DM, Basler JW, et al. Percutaneous Management of Urolithiasis During Pregnancy. J Urol 1992; 148(3):1069-1071.
17. Joshi HB, Adams S, Obadeyi OO, et al. Nephrostomy tube or “JJ” ureteric stent in ureteric obstruction: assessment of patient perspectives using quality of life survey and utility analysis. Eur Urol 2001; 39:695-701.
18. Mokhmalji H, Braun PM, Portillo JM, et al. Percutaneous nephrostomy versus ureteral stents for diversion of hydronephrosis caused by stones: a prospective randomized clinical trial. J Urol 2001; 165:1088-1092.
19. Mazze RI, Kallen B. Reproductive outcome after anesthesia and operation during pregnancy: a registry study of 5405 cases. Amer J Obst Gynec 1989, 161:1178-1185.
20. Goldfarb R, Nerrhut G, Lederer E. Management of acute hydronephrosis of pregnancy by ureteral stenting: risk of stone formation. J Urol 1989; 141:921-922.

 

Date added to bjui.org: 08/12/2012

DOI: 10.1002/BJUIw-2012-058-web

 

Non-Hodgkin’s lymphoma presenting as macroscopic haematuria and acute urinary retention in pregnancy

We present the case of a 30 year old primigravida in her third trimester with visible haematuria secondary to a genital-tract lymphoma. 

 

Authors: Mark Sayles1, Sarah E Gull2, James DD Allan1

1. Department of Urology
West Suffolk Hospital
Hardwick Lane
Bury St Edmunds
Suffolk
IP33 2QZ
2. Department of Obstetrics and Gynaecology
West Suffolk Hospital
Hardwick Lane
Bury St Edmunds
Suffolk
IP33 2QZ

 
Corresponding Author: Mark Sayles, Department of Urology, West Suffolk Hospital, Hardwick Lane, Bury St Edmunds, Suffolk. E-mail: [email protected]

 

Abbreviations
MRI  Magnetic Resonance Imaging
R-CHOP  Rituximab-(Cyclophosphamide, Doxorubicin, Vincristine, Prednisolone)
PCR  Polymerase Chain Reaction
FISH   Flourescent In-Situ Hybridisation
DLBCL   Diffuse Large B-Cell Lymphoma

 

Introduction
 
Non-Hodgkin’s lymphoma of the female genital tract during pregnancy is rare. We present the case of a 30 year old primigravida in her third trimester with visible haematuria secondary to a genital-tract lymphoma. We describe the management of this unusual occurrence and review the relevant literature.

 

Case Report
 
A 30 year old primigravida presented at 29 weeks gestation with macroscopic haematuria and intermittent acute urinary retention associated with blood clots. She had no significant past medical history, and the early pregnancy had been largely unremarkable. Three months earlier she had been treated with oral antibiotics for a presumed urinary tract infection, having presented to her general practitioner with haematuria. She was taking iron supplements because of anaemia noted at a routine antenatal check (haemoglobin 7g/dL).
Ultrasound examination revealed bilateral hydronephrosis and a complex solid mass on the posterior bladder wall. She underwent pelvic MRI, and urgent flexible cystoscopy, vaginal examination, and biopsy under general anaesthesia. MRI showed a 10cm x 8cm irregular soft-tissue mass involving the anterior vaginal wall and fornix, the proximal urethra, and the posterior bladder wall (Figure 1).

 

Figure 1. T2-weighted magnetic resonance images of the abdomen and pelvis in coronal (A) and sagittal (B) section. Note the large mass invading into the bladder, and the proximity of tumour to the gravid uterus.

 

 

The tumour extended into the bladder, and the bilateral hydronephrosis seen on ultrasound imaging was due to involvement of both distal ureters by tumour.
Cystoscopy and examination revealed a partially fixed mass, between the anterior vaginal wall and the bladder, which was invading the trigone. Biopsies were taken from the mass in the bladder and from the portion in the vaginal wall. These were submitted for detailed immunohistochemical analysis.
On microscopy the pathological specimens showed a diffuse infiltrate of large atypical lymphoid cells. Immunostaining demonstrated expression of CD20, BCL2, CD30 and CD5, with a proportion of cells expressing MUM-1. PCR analysis detected clonal IgH and IgΚ rearrangements, while FISH showed no evidence of IgH, BCL2, BCL6, or MYC translocation but detected gain of extra copies of each of these gene loci. In summary, the immunohistochemical analyses were consistent with a diagnosis of diffuse large B cell lymphoma (DLBCL).
After multidisciplinary discussion, it was decided to commence R-CHOP chemotherapy. The fetus was delivered at 35 weeks gestation by classical Caesarian section, between the first and second cycles of chemotherapy. Both mother and fetus tolerated the first cycle of chemotherapy well, and a 2.6 kg boy was born at 35 weeks gestation as planned. The boy spent one week in a special care baby unit because of prematurity, but required no specific isolation measures. The patient’s tumour responded well to chemotherapy, and she remains under long-term follow up.

 

Discussion
 
Cancer complicates approximately 1:1000 pregnancies [1]. Lymphoma is the fourth most commonly diagnosed malignancy in pregnancy, complicating approximately 1:6000 deliveries [2]. The majority of lymphomas in women of childbearing age are Hodgkin’s disease, with Non-Hodgkin’s lymphoma (NHL) being much rarer in this age group [2, 3]. When NHL does occur in pregnancy, it tends to be of an aggressive subtype such as DLBCL [4]. In general, primary extranodal disease occurs in 20-30% of lymphoma cases, most frequently involving the gastrointestinal tract or skin [5]. Only 0.5% of extranodal lymphomas originate in the female genital tract [6].
DLBCL is associated with an aggressive natural history; median survival in untreated patients is less than one year [7]. Treatment with a combination chemotherapy regime (Cyclophosphamide, Doxorubicin, Vincristine, and Prednisolone; CHOP) has been standard practice for several decades [8]. The addition of rituximab (a chimeric IgG1κ anti-CD20 monoclonal antibody) to this regime (R-CHOP) significantly reduces the risk of relapse and improves overall survival [9, 10].
Because the diagnosis of cancer during pregnancy is a relatively rare event, experience with the use of chemotherapeutic agents and monoclonal antibodies in pregnancy is limited [1, 2, 11]. Almost all chemotherapeutic agents are known to be teratogenic in animals, and to lead to major malformations or fetal death if administered during the first trimester [12, 13]. Exposure during the second and third trimester is not associated with fetal malformations, but increases the risk of fetal and neonatal death, intrauterine growth retardation, preterm delivery and low birth weight [14]. Clearly, the decision to undergo chemotherapy during pregnancy is complex, and has to balance the risks and benefits to both mother and fetus.
Importantly, there are no preclinical reproductive toxicity data available for rituximab. There are only seven previously documented cases of rituximab being used for lymphoma in pregnancy [15-21]. In the majority of these cases rituximab was administered during the second trimester for an aggressive NHL [15-20]. In one case the patient was taking a maintenance dose of rituximab for relapsing follicular lymphoma during which she conceived unintentionally [21]. The rituximab was stopped and the pregnancy continued to term. Of the seven children exposed to rituximab in utero, three were found to have severely decreased CD19+ B cells as neonates. However, none experienced any significant postnatal infection, and in each case the level of B cells returned to normal within three to six months.

 

Conclusion
 
Rare cancers in pregnancy present diagnostic and therapeutic challenges. The successful outcome in this case required a multidisciplinary approach involving obstetrics and gynaecology, urology, and oncology specialist input. This is the eighth documented case of a fetus being exposed to rituximab, without apparent short term clinically significant effects. However, the long term effects of this exposure are unknown.

 

References
[1] Pentheroudakis G, Pavlidis N. Cancer and pregnancy: poena magna, not anymore. Eur J Cancer. 2006 Jan: 42:126-40
[2] Pavlidis NA. Coexistence of pregnancy and malignancy. Oncologist. 2002: 7:279-87
[3] Ward FT, Weiss RB. Lymphoma and pregnancy. Semin Oncol. 1989 Oct: 16:397-409
[4] Lishner M, Zemlickis D, Sutcliffe SB, Koren G. Non-Hodgkin’s lymphoma and pregnancy. Leuk Lymphoma. 1994 Aug: 14:411-3
[5] Krol AD, le Cessie S, Snijder S, Kluin-Nelemans JC, Kluin PM, Noordijk EM. Primary extranodal non-Hodgkin’s lymphoma (NHL): the impact of alternative definitions tested in the Comprehensive Cancer Centre West population-based NHL registry. Ann Oncol. 2003 Jan: 14:131-9
[6] Lagoo AS, Robboy SJ. Lymphoma of the female genital tract: current status. Int J Gynecol Pathol. 2006 Jan: 25:1-21
[7] Fisher RI, Miller TP, O’Connor OA. Diffuse aggressive lymphoma. Hematology Am Soc Hematol Educ Program. 2004:221-36
[8] Flowers CR, Sinha R, Vose JM. Improving outcomes for patients with diffuse large B-cell lymphoma. CA Cancer J Clin. 2010 Nov-Dec: 60:393-408
[9] Coiffier B, Lepage E, Briere J, et al. CHOP chemotherapy plus rituximab compared with CHOP alone in elderly patients with diffuse large-B-cell lymphoma. N Engl J Med. 2002 Jan 24: 346:235-42
[10] Pfreundschuh M, Trumper L, Osterborg A, et al. CHOP-like chemotherapy plus rituximab versus CHOP-like chemotherapy alone in young patients with good-prognosis diffuse large-B-cell lymphoma: a randomised controlled trial by the MabThera International Trial (MInT) Group. Lancet Oncol. 2006 May: 7:379-91
[11] Azim HA, Jr., Azim H, Peccatori FA. Treatment of cancer during pregnancy with monoclonal antibodies: a real challenge. Expert Rev Clin Immunol. 2010 Nov: 6:821-6
[12] Cardonick E, Iacobucci A. Use of chemotherapy during human pregnancy. Lancet Oncol. 2004 May: 5:283-91
[13] Leslie KK, Koil C, Rayburn WF. Chemotherapeutic drugs in pregnancy. Obstet Gynecol Clin North Am. 2005 Dec: 32:627-40
[14] Weisz B, Meirow D, Schiff E, Lishner M. Impact and treatment of cancer during pregnancy. Expert Rev Anticancer Ther. 2004 Oct: 4:889-902
[15] Cordeiro A, Machado AI, Borges A, Alves MJ, Frade MJ. Burkitt’s lymphoma related to Epstein-Barr virus infection during pregnancy. Arch Gynecol Obstet. 2009 Aug: 280:297-300
[16] Rey J, Coso D, Roger V, et al. Rituximab combined with chemotherapy for lymphoma during pregnancy. Leuk Res. 2009 Mar: 33:e8-9
[17] Decker M, Rothermundt C, Hollander G, Tichelli A, Rochlitz C. Rituximab plus CHOP for treatment of diffuse large B-cell lymphoma during second trimester of pregnancy. Lancet Oncol. 2006 Aug: 7:693-4
[18] Magloire LK, Pettker CM, Buhimschi CS, Funai EF. Burkitt’s lymphoma of the ovary in pregnancy. Obstet Gynecol. 2006 Sep: 108:743-5
[19] Friedrichs B, Tiemann M, Salwender H, Verpoort K, Wenger MK, Schmitz N. The effects of rituximab treatment during pregnancy on a neonate. Haematologica. 2006 Oct: 91:1426-7
[20] Herold M, Schnohr S, Bittrich H. Efficacy and safety of a combined rituximab chemotherapy during pregnancy. J Clin Oncol. 2001 Jul 15: 19:3439
[21] Kimby E, Sverrisdottir A, Elinder G. Safety of rituximab therapy during the first trimester of pregnancy: a case history. Eur J Haematol. 2004 Apr: 72:292-5

 

Date added to bjui.org: 15/11/2011 


DOI: 10.1002/BJUIw-2011-061-web

 

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